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H3 K27-altered diffuse midline glioma presenting as massive cerebellopontine hemorrhage

Tej D Azad1, Anita L Kalluri1, Landon J Hansen1

  • 1Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|March 3, 2023
PubMed

Insights

A pediatric case highlights that diffuse midline glioma (WHO grade 4) can present as extensive hemorrhage, even without identified vascular malformations. This emphasizes the need to investigate glioma as a cause of unexplained hemorrhage in children.

Area of Science:

  • Pediatric Neurosurgery
  • Neuro-oncology
  • Neuropathology

Background:

  • Arteriovenous malformations (AVMs) are a common cause of hemorrhage in children.
  • Diffuse midline gliomas (WHO grade 4) are aggressive brain tumors typically diagnosed in children.

Observation:

  • A 14-year-old boy presented with massive cerebellar and brainstem hemorrhage.
  • Initial cerebral angiograms did not reveal any vascular abnormalities.
  • Surgical evacuation of the hematoma allowed for pathological analysis.

Findings:

  • Pathology confirmed diffuse midline glioma, H3 K27-altered (WHO grade 4).
  • The patient rapidly developed diffuse craniospinal leptomeningeal disease.
  • Neurologic decline and respiratory failure occurred without further hemorrhage.

Implications:

  • This case underscores the importance of considering diffuse midline glioma in the differential diagnosis of pediatric intracranial hemorrhage when vascular etiologies are absent.
  • Early recognition and further research into H3 K27-altered gliomas are crucial for improved patient outcomes.
Keywords:
Case reportH3 K27-altered diffuse midline gliomaIntracranial hemorrhage

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